Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome.
Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome.
复制标题
DOI：
10.1016/j.cub.2009.10.074
复制
发表时间：
2009-12-29
期刊：
影响因子：
--
通讯作者：
Pfanner N
中科院分区：
文献类型：
--
作者：
Gebert N;Joshi AS;Kutik S;Becker T;McKenzie M;Guan XL;Mooga VP;Stroud DA;Kulkarni G;Wenk MR;Rehling P;Meisinger C;Ryan MT;Wiedemann N;Greenberg ML;Pfanner N
The biogenesis of mitochondria requires the import of a large number of proteins from the cytosol. While numerous studies have defined the proteinaceous machineries that mediate mitochondrial protein sorting, little is known about the role of lipids in mitochondrial protein import. Cardiolipin, the signature phospholipid of the mitochondrial inner membrane, affects the stability of many inner membrane protein complexes. Perturbation of cardiolipin metabolism leads to the X-linked cardioskeletal myopathy, Barth syndrome. We report that cardiolipin affects the preprotein translocases of the mitochondrial outer membrane. Cardiolipin mutants genetically interact with mutants of outer membrane translocases. Mitochondria from cardiolipin yeast mutants, as well as Barth syndrome patients, are impaired in the biogenesis of outer membrane proteins. Our findings reveal a new role for cardiolipin in protein sorting at the mitochondrial outer membrane and bear implications for the pathogenesis of Barth syndrome.
登录
查看更多内容
影响因子：
30.8
作者：
Bione, S;DAdamo, P;Toniolo, D
通讯作者：
Toniolo, D
影响因子：
3.3
作者：
Kim, TH;Zhao, Y;Yin, XM
通讯作者：
Yin, XM
影响因子：
4.8
作者：
Pfeiffer, K;Gohil, V;Schägger, H
通讯作者：
Schägger, H
影响因子：
3.4
作者：
deKroon, AIPM;Dolis, D;deKruijff, B
通讯作者：
deKruijff, B
影响因子：
64.5
作者：
Chacinska, A;Lind, M;Rehling, P
通讯作者：
Rehling, P